Objectives This study sought to examine the impact of surgical timing on major morbidity and hospital reimbursement for late preterm and term infants with dextrotransposition of the great arteries ...(d-TGA). Background Neonatal arterial switch operation is the standard of care for d-TGA. Little is known about the effects of age at operation on clinical outcomes or costs for these neonates. Methods We conducted a retrospective cohort study of infants at ≥36 weeks' gestation, with d-TGA, with or without ventricular septal defects, admitted to our institution at 5 days of age or younger, between January 1, 2003 and October 1, 2012. Children with other cardiac abnormalities or other major comorbid conditions were excluded. Univariable and multivariable analyses were performed to determine the effects of age at operation on major morbidity and hospital reimbursement. Results A total of 140 infants met inclusion criteria. Reimbursement data were available for them through January 1, 2012 (n = 128). The mortality rate was 1.4% (n = 2). Twenty percent (n = 28) experienced a major morbidity. The median costs were $60,000, in 2012 dollars (range: $25,000 to $549,000). The median age at operation was 5 days (range: 1 to 12 days). For every day later that surgery was performed, beyond day of life 3, the odds of major morbidity increased by 47% (range: 23% to 66%, p < 0.001) and costs increased by 8% (range: 5% to 11%, p < 0.001), after considering the effects of sex, birth weight, gestational age, year at which surgery was performed, transfer, weekend admission, insurance, surgeon, septostomy, bypass and cross-clamp times, and the presence of ventricular septal defects or abnormal coronary anatomy. Conclusions Delay of neonatal arterial switch operation beyond 3 days is significantly associated with increased morbidity and healthcare costs.
Objective Two randomized trials of hematocrit strategy during hypothermic cardiopulmonary bypass in infant heart surgery have been performed. The first suggested worse outcomes were concentrated in ...patients with lower hematocrit levels (approximately 20%), whereas the second suggested there was little benefit to increasing the hematocrit level above 25%. The form of the relationship between continuous hematocrit levels and outcomes requires further study. Methods In the two trials, 271 infants who underwent biventricular repair not involving the aortic arch were enrolled. Analysis was undertaken of the effects of hematocrit level, as a continuous variable, at the onset of low-flow cardiopulmonary bypass. Results Psychomotor Development Index scores at age 1 year varied nonlinearly with hematocrit levels, with increasing scores up to 23.5% hematocrit ( P < .001) and a plateau effect beyond 23.5% ( P = .42), based on a piecewise linear model. Lower hematocrit levels were associated with more positive intraoperative fluid balance ( P < .001 for linear trend) and marginally associated with higher serum lactate levels at 60 minutes after bypass ( P = .08 for linear trend), but not with blood products given, nadir of cardiac index in the first 24 hours, or Mental Development Index scores. Conclusions A hematocrit level at the onset of low-flow cardiopulmonary bypass of approximately 24% or higher is associated with higher Psychomotor Development Index scores and reduced lactate levels. Because the effects of hemodilution may vary according to diagnosis, age at operation, bypass variables such as pH strategy and flow rate, and other perioperative factors, this study cannot ascertain a universally “safe” hemodilution level.
Red blood cell transfusion is associated with morbidity and mortality among adults undergoing cardiac surgery. We aimed to evaluate the association of transfusion with morbidity among pediatric ...cardiac surgical patients.
Patients discharged after cardiac surgery in 2003 were retrospectively reviewed. The red blood cell volume administered during the first 48 postoperative hours was used to classify patients into nonexposure, low exposure (≤15 mL/kg), or high exposure (>15 mL/kg) groups. Cox proportional hazards modeling was used to evaluate the association of red blood cell exposure to length of hospital stay (LOS).
Of 802 discharges, 371 patients (46.2%) required blood transfusion. Demographic differences between the transfusion exposure groups included age, weight, prematurity, and noncardiac structural abnormalities (all p<0.001). Distribution of Risk Adjusted Classification for Congenital Heart Surgery, version 1 (RACHS-1) categories, intraoperative support times, and postoperative Pediatric Risk of Mortality Score, Version III (PRISM-III) scores varied among the exposure groups (p<0.001). Median duration of mechanical ventilation (34 hours 0 to 493 versus 27 hours 0 to 621 versus 16 hours 0 to 375), incidence of infection (21 14% versus 29 13% versus 17 4%), and acute kidney injury (25 17% versus 29 13% versus 34 8%) were highest in the high transfusion exposure group when compared with the low or nontransfusion groups (all p<0.001). In a multivariable Cox proportional hazards model, both the low transfusion group (adjusted hazard ratio HR 0.80, 95% confidence interval CI: 0.66 to 0.97, p=0.02) and high transfusion group (adjusted HR 0.66, 95% CI: 0.53 to 0.82, p<0.001) were associated with increased LOS. In subgroup analyses, both low transfusion (adjusted HR 0.81, 95% CI: 0.65 to 1.00, p=0.05) and high transfusion (adjusted HR 0.65, 95% CI: 0.49 to 0.87, p=0.004) in the biventricular group but not in the single ventricle group was associated with increased LOS.
Blood transfusion is associated with prolonged hospitalization of children after cardiac surgery, with biventricular patients at highest risk for increased LOS. Future studies are necessary to explore this association and refine transfusion practices.
Objective Borderline left heart disease is characterized by left heart obstructive lesions (coarctation, aortic and mitral stenoses, left ventricular hypoplasia) and endocardial fibroelastosis. The ...multilevel obstruction and impaired left ventricular systolic and diastolic function contribute to failure of biventricular circulation. We studied the effects of left ventricular rehabilitation—endocardial fibroelastosis resection with mitral or aortic valvuloplasty—on left ventricular function and clinical outcomes. Methods All patients with borderline left heart structures and endocardial fibroelastosis who underwent a primary left ventricular rehabilitation procedure were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Left heart dimensions and hemodynamics were recorded from preoperative and postoperative echocardiogram and cardiac catheterization. Postoperative left atrial pressure was obtained from the intracardiac line early after left ventricular rehabilitation. Preoperative and postoperative values were compared by paired t test. Results Between 1999 and 2008, 9 patients with endocardial fibroelastosis and borderline left heart disease underwent left ventricular rehabilitation at a median age of 5.6 months (range, 1–38 months). There was no operative mortality, and at a median follow-up of 25 months (6 months to 10 years) there was 1 death from noncardiac causes and 2 patients required reoperations. Significant increases in ejection fraction and left ventricular end-diastolic volume were observed, whereas left atrial pressure and right ventricular/left ventricular pressure ratios decreased postoperatively. Conclusion In patients with borderline left hearts, primary left ventricular rehabilitation with endocardial fibroelastosis resection and mitral and aortic valvuloplasty results in improved left ventricular systolic and diastolic performance and decreased right ventricular pressures. This approach may provide an alternative to single-ventricle management in this difficult patient group.
Objectives Continuous flow in the Fontan circulation results in impairment of pulmonary artery endothelial function, increased pulmonary arterial resistance, and, potentially, late failure of Fontan ...circulation. We investigated the mechanisms of vascular remodeling and altered vascular reactivity associated with chronic privation of pulsatility on pulmonary vasculature. Methods A total of 30 pigs were evenly distributed in 3 groups: 10 underwent a sham procedure (group I) and 20 underwent a cavopulmonary shunt between the superior vena cava and right pulmonary artery—10 with complete ligation of the proximal right pulmonary artery (group II, nonpulsatile) and 10 with partial ligation (group III, micropulsatile). At 3 months postoperatively, the in vivo hemodynamics, in vitro vasomotricity (concentration response curves on pulmonary artery isolated rings), and endothelial nitric oxide synthase protein level were assessed. A comparison between group and between the right and left lung in each group was performed. Results Group II developed right pulmonary hypertension and increased right pulmonary resistance. Endothelial function was altered in group II, as reflected by a decrease in the vasodilation response to acetylcholine and ionophoric calcium but preservation of the nonendothelial-dependent response to sodium nitroprusside. Group III micropulsatility attenuated pulmonary hypertension but did not prevent impairment of the endothelial-dependant relaxation response. Right lung Western blotting revealed decreased endothelial nitric oxide synthase in group II (0.941 ± 0.149 vs sham 1.536 ± 0.222, P = .045) that was preserved in group III (1.275 ± 0.236, P = .39). Conclusions In a chronic model of unilateral cavopulmonary shunt, pulsatility loss resulted in an altered endothelial-dependant vasorelaxation response of the pulmonary arteries. Micropulsatility limited the effects of pulsatility loss. These results are of importance for potential therapies against pulmonary hypertension in the nonpulsatile Fontan circulation, by retaining accessory pulmonary flow or pharmaceutical modulation of nonendothelial-dependant pulmonary vasorelaxation.
Objectives The Ross procedure is used to treat aortic valve disease in children. The advantages include autograft growth, long-term durability, and avoidance of anticoagulation. Long-term follow-up ...of the Ross procedure in infancy is limited. We sought to characterize the long-term outcomes of infants undergoing the Ross procedure. Methods We performed a retrospective review of all patients who underwent a Ross operation at 18 months of age or younger at New-York Presbyterian and Cardiothoracic Center of Monaco from 1991 to 2010. The clinical, catheterization, and surgical records were reviewed. The most recent follow-up information, including echocardiogram and electrocardiogram, was obtained and analyzed. Results A total of 34 patients underwent a Ross procedure at a median age of 6 months (range, 4 days to 18.4 months). All had congenital aortic stenosis. All but 1 patient had undergone previous surgical or catheter-based interventions. The median follow-up was 10.6 years (range, 1.4-20.4 years). There were 4 early deaths and 1 late transplant. The freedom from right ventricular outflow tract reintervention was 85% at 5 years and 64% at 10 years. The freedom from autograft reintervention was 95.5% at 10 years. In 20 subjects, late follow-up echocardiograms showed a significant difference between the mean early and late Z scores of the autograft annulus (0.8 vs 2.4, P = .03), sinus (0.8 vs 2.8, P = .002), and sinotubular junction (1.2 vs 2.7, P = .04). Mild or less aortic insufficiency occurred in 17 subjects. None had significant aortic stenosis. Conclusions The long-term outcomes of the Ross procedure in infants and toddlers are favorable despite moderate dilatation of the autograft. Reintervention at the right ventricular outflow tract is common.
Objective We sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure. Methods From January 2001 through ...December 2006, all patients with hypoplastic left heart syndrome who underwent stage I palliation at Children’s Hospital Boston were retrospectively reviewed. The subgroup with the mitral stenosis–aortic atresia variant was studied separately. We evaluated preoperative echocardiographic data, operative characteristics, and postoperative factors associated with death or the need for transplantation. The Kaplan–Meier method was used to assess survival. Results Thirty-eight (23%) of 165 patients had mitral stenosis–aortic atresia. Hospital mortality or need for transplantation for patients with mitral stenosis–aortic atresia was significantly higher than for other anatomic subgroups (29% vs 7.9%, P = .002). Left ventricle–subepicardial coronary artery communications were present in 20 (53%) patients with mitral stenosis–aortic atresia and were associated with a significantly higher hospital mortality (50% vs 6%, P = .004). No difference in outcome was demonstrated between different sources of pulmonary blood flow. A longer cardiopulmonary bypass time ( P = .02) and the need for postoperative extracorporeal membrane oxygenation support ( P < .001) were associated with a higher mortality rate. Conclusions With improved outcomes in the management of neonates with hypoplastic left heart syndrome, those with the mitral stenosis–aortic atresia variant and left ventricle–subepicardial coronary artery fistulae have emerged as a higher-risk subgroup for failure of stage I palliation. Further investigation is required, and a change in clinical management strategy for this particular subgroup might be warranted.
Abstract The time interval for the doubling of medical knowledge continues to decline. Physicians, patients, administrators, government officials, and payors are struggling to keep up to date with ...the waves of new information and to integrate the knowledge into new patient treatment protocols, processes, and metrics. Guidelines, Consensus Guidelines, and Consensus Statements, moderated by seasoned content experts, offer one method to rapidly distribute new information in a timely manner and also guide minimal standards of treatment of clinical care pathways as they are developed as part of bundled care programs. These proposed Consensus Guidelines advance The American Association for Thoracic Surgery's mission of leading in cardiothoracic health care, education, innovation, and modeling excellence.
Optimal timing for total repair in tetralogy of Fallot (TOF) is controversial. We aimed to determine if weight at 1 year differs between patients who undergo neonatal total repair versus those who ...undergo nonneonatal total repair later in the first year of life.
A retrospective review of infants admitted with TOF between January 2004 and June 2011 was conducted. Patient data, including weight, were collected throughout the first year of life, and neonatal total repair versus nonneonatal total repair groups were compared.
Of 163 infants, neonatal total repair was undertaken in 36 (22%) of them, whereas 127 (78%) infants had nonneonatal total repair at greater than 28 days of life. The median neonatal intensive care unit length of stay (LOS) was longer for the neonatal total repair group than for the nonneonatal total repair group (17.5 11-24 versus 7 0-15 days; p < 0.001). Patients in the neonatal total repair group were more likely to have a transannular patch (TAP) (p < 0.001) than were those in the nonneonatal total repair group, whereas patients in the nonneonatal total repair group were more likely to have undergone a valve-sparing operation (p = 0.002). The mean weight-for-age z score was 0.7 higher in the neonatal total repair group compared with the nonneonatal total repair group (p = 0.03) controlling for birth weight (BW), diagnostic subgroup, and gestational age (GA).
Patients with TOF who underwent neonatal total repair were more likely to receive a TAP but had higher weight-for-age scores at 1 year compared with patients who underwent full repair later in the first year of life.
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